PBC1
MCID: BLR024
MIFTS: 30

Biliary Cirrhosis, Primary, 1 (PBC1)

Categories: Gastrointestinal diseases, Genetic diseases, Immune diseases, Liver diseases, Rare diseases

Aliases & Classifications for Biliary Cirrhosis, Primary, 1

MalaCards integrated aliases for Biliary Cirrhosis, Primary, 1:

Name: Biliary Cirrhosis, Primary, 1 56 13
Primary Biliary Cirrhosis 71
Pbc1 56
Pbc 56

Characteristics:

OMIM:

56
Inheritance:
autosomal dominant


HPO:

31
biliary cirrhosis, primary, 1:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

OMIM 56 109720
OMIM Phenotypic Series 56 PS109720
MedGen 41 C4551595
SNOMED-CT via HPO 68 1761006 263681008 31712002
UMLS 71 C0008312

Summaries for Biliary Cirrhosis, Primary, 1

OMIM : 56 Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic liver disease that usually affects middle-aged women and eventually leads to liver failure (summary by Kaplan, 1996). (109720)

MalaCards based summary : Biliary Cirrhosis, Primary, 1, also known as primary biliary cirrhosis, is related to biliary cirrhosis, primary, 2 and reynolds syndrome. The drugs Abatacept and Sorafenib have been mentioned in the context of this disorder. Affiliated tissues include liver, b cells and bone marrow, and related phenotype is biliary cirrhosis.

Related Diseases for Biliary Cirrhosis, Primary, 1

Diseases in the Primary Biliary Cirrhosis family:

Biliary Cirrhosis, Primary, 1 Biliary Cirrhosis, Primary, 2
Biliary Cirrhosis, Primary, 3 Biliary Cirrhosis, Primary, 4
Biliary Cirrhosis, Primary, 5

Diseases related to Biliary Cirrhosis, Primary, 1 via text searches within MalaCards or GeneCards Suite gene sharing:

(showing 76, show less)
# Related Disease Score Top Affiliating Genes
1 biliary cirrhosis, primary, 2 11.6
2 reynolds syndrome 11.6
3 primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome 11.3
4 biliary cirrhosis, primary, 3 11.2
5 biliary cirrhosis, primary, 4 11.2
6 biliary cirrhosis, primary, 5 11.2
7 nodular regenerative hyperplasia 11.2
8 liver cirrhosis 10.9
9 cholangitis 10.8
10 liver disease 10.6
11 autoimmune hepatitis 10.6
12 cholangitis, primary sclerosing 10.5
13 sclerosing cholangitis 10.5
14 autoimmune disease 10.5
15 varicose veins 10.4
16 scleroderma, familial progressive 10.4
17 raynaud phenomenon 10.4
18 portal hypertension 10.3
19 esophageal varix 10.3
20 telangiectasis 10.2
21 primary biliary cirrhosis 10.2
22 cholestasis 10.2
23 crest syndrome 10.2
24 viral hepatitis 10.2
25 systemic scleroderma 10.2
26 systemic lupus erythematosus 10.1
27 sjogren syndrome 10.1
28 cholangiocarcinoma 10.1
29 calcinosis 10.1
30 intrahepatic cholangiocarcinoma 10.1
31 cytokine deficiency 10.1
32 pfeiffer syndrome 9.9
33 myositis 9.9
34 osteoporosis 9.9
35 ovarian cancer 9.9
36 alkaline phosphatase, plasma level of, quantitative trait locus 1 9.9
37 sarcoidosis 1 9.9
38 thrombocytopenic purpura, autoimmune 9.9
39 trigeminal neuralgia 9.9
40 proteasome-associated autoinflammatory syndrome 1 9.9
41 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
42 bone mineral density quantitative trait locus 8 9.9
43 bone mineral density quantitative trait locus 15 9.9
44 graft-versus-host disease 9.9
45 leukemia, acute lymphoblastic 3 9.9
46 helix syndrome 9.9
47 chlamydia pneumonia 9.9
48 intrahepatic cholestasis of pregnancy 9.9
49 bone resorption disease 9.9
50 non-alcoholic steatohepatitis 9.9
51 sialadenitis 9.9
52 chlamydia 9.9
53 neutropenia 9.9
54 graves' disease 9.9
55 acute cystitis 9.9
56 renal tubular acidosis 9.9
57 hepatitis b 9.9
58 bilirubin metabolic disorder 9.9
59 antiphospholipid syndrome 9.9
60 purpura 9.9
61 arthropathy 9.9
62 hepatitis e 9.9
63 severe combined immunodeficiency 9.9
64 connective tissue disease 9.9
65 thyroiditis 9.9
66 active peptic ulcer disease 9.9
67 muscular atrophy 9.9
68 collagen disease 9.9
69 viral infectious disease 9.9
70 fatty liver disease 9.9
71 noma 9.9
72 hypereosinophilic syndrome 9.9
73 chronic graft versus host disease 9.9
74 distal renal tubular acidosis 9.9
75 paresthesia 9.9
76 argyria 9.9

Graphical network of the top 20 diseases related to Biliary Cirrhosis, Primary, 1:



Diseases related to Biliary Cirrhosis, Primary, 1

Symptoms & Phenotypes for Biliary Cirrhosis, Primary, 1

Human phenotypes related to Biliary Cirrhosis, Primary, 1:

31 (showing 1, show less)
# Description HPO Frequency HPO Source Accession
1 biliary cirrhosis 31 HP:0002613

Symptoms via clinical synopsis from OMIM:

56
G I:
primary biliary cirrhosis

Lab:
impaired in vitro igg suppression

Clinical features from OMIM:

109720

Drugs & Therapeutics for Biliary Cirrhosis, Primary, 1

Drugs for Biliary Cirrhosis, Primary, 1 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(showing 111, show less)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Abatacept Approved Phase 4 332348-12-6 10237
2
Sorafenib Approved, Investigational Phase 4 284461-73-0 216239 406563
3 Immunologic Factors Phase 4
4 Immunosuppressive Agents Phase 4
5 Antirheumatic Agents Phase 4
6 Liver Extracts Phase 4
7 Protein Kinase Inhibitors Phase 4
8
Fenofibrate Approved Phase 3 49562-28-9 3339
9
Metformin Approved Phase 2, Phase 3 657-24-9 14219 4091
10
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
11
chenodeoxycholic acid Approved Phase 3 474-25-9 10133
12
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
13
leucovorin Approved Phase 3 58-05-9 6006 143
14
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
15
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
16
Colchicine Approved Phase 3 64-86-8 2833 6167
17
Copper Approved, Investigational Phase 3 7440-50-8 27099
18
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
19
Atorvastatin Approved Phase 3 134523-00-5 60823
20
Ursodeoxycholic acid Approved, Investigational Phase 3 128-13-2 31401
21
Budesonide Approved Phase 3 51333-22-3 63006 5281004
22
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
23
Tauroursodeoxycholic acid Experimental, Investigational Phase 3 14605-22-2 12443252
24
Tetrathiomolybdate Investigational Phase 3 16330-92-0
25 Hypoglycemic Agents Phase 2, Phase 3
26 Laxatives Phase 3
27 Cathartics Phase 3
28 Anti-Infective Agents Phase 3
29 Vitamin B Complex Phase 3
30 Dermatologic Agents Phase 3
31 Folic Acid Antagonists Phase 3
32 Folate Phase 3
33 Vitamin B9 Phase 3
34 Lamivudine, zidovudine drug combination Phase 2, Phase 3
35 Tubulin Modulators Phase 3
36 Antimitotic Agents Phase 3
37 Angiogenesis Inhibitors Phase 3
38 Chelating Agents Phase 3
39 Copper Supplement Phase 3
40 Lipid Regulating Agents Phase 3
41 Hypolipidemic Agents Phase 3
42 Antimetabolites Phase 3
43 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
44 Anticholesteremic Agents Phase 3
45 carnitine Phase 3
46 Respiratory System Agents Phase 3
47 Hormones Phase 3
48 Anti-Asthmatic Agents Phase 3
49 Hormone Antagonists Phase 3
50 glucocorticoids Phase 3
51 Bronchodilator Agents Phase 3
52 Anti-Inflammatory Agents Phase 3
53
Sertraline Approved Phase 2 79617-96-2 68617
54
Pentoxifylline Approved, Investigational Phase 2 6493-05-6 4740
55
Moexipril Approved Phase 2 103775-10-6 91270
56
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
57
Ustekinumab Approved, Investigational Phase 2 815610-63-0
58
Entecavir Approved, Investigational Phase 2 142217-69-4 153941
59
Rifaximin Approved, Investigational Phase 2 80621-81-4 6436173 46783403
60
Methionine Approved, Nutraceutical Phase 2 63-68-3 6137
61
Tenofovir Experimental, Investigational Phase 2 147127-20-6 464205
62
Bilirubin Phase 1, Phase 2 69853-43-6, 635-65-4 21252250 5280352
63 Clofibric Acid Phase 1, Phase 2 882-09-7
64 Serotonin Uptake Inhibitors Phase 2
65 Psychotropic Drugs Phase 2
66 Neurotransmitter Agents Phase 2
67 Antidepressive Agents Phase 2
68
protease inhibitors Phase 2
69 HIV Protease Inhibitors Phase 2
70 Phosphodiesterase Inhibitors Phase 2
71 Antioxidants Phase 2
72 Vasodilator Agents Phase 2
73 Platelet Aggregation Inhibitors Phase 2
74 Protective Agents Phase 2
75 Radiation-Protective Agents Phase 2
76 Antihypertensive Agents Phase 2
77 Angiotensin-Converting Enzyme Inhibitors Phase 2
78 Antineoplastic Agents, Immunological Phase 1, Phase 2
79 Anti-Bacterial Agents Phase 2
80 Adrenergic beta-Antagonists Phase 2
81 Antibiotics, Antitubercular Phase 2
82 Immunoglobulins Phase 2
83 Antibodies Phase 2
84 Antibodies, Monoclonal Phase 2
85
Serotonin Investigational, Nutraceutical Phase 2 50-67-9 5202
86
Tigecycline Approved Phase 1 220620-09-7 5282044
87
Modafinil Approved, Investigational Phase 1 68693-11-8 4236
88
Adenosine Approved, Investigational Phase 1 58-61-7 60961
89
Trichostatin A Experimental Phase 1 58880-19-6
90 Immunoglobulins, Intravenous Phase 1
91 Immunoglobulin G Phase 1
92 Immunoglobulin M Phase 1
93 Central Nervous System Stimulants Phase 1
94 Pharmaceutical Solutions Phase 1
95
Pioglitazone Approved, Investigational 111025-46-8 4829
96
Lopinavir Approved 192725-17-0 92727
97
Emtricitabine Approved, Investigational 143491-57-0 60877
98
Ritonavir Approved, Investigational 155213-67-5 392622
99
Vitamin D Approved, Nutraceutical, Vet_approved 1406-16-2
100 Gastrointestinal Agents Early Phase 1
101 Cytochrome P-450 Enzyme Inhibitors
102 Anti-HIV Agents
103 Reverse Transcriptase Inhibitors
104 Emtricitabine, Tenofovir Disoproxil Fumarate Drug Combination
105 Cytochrome P-450 CYP3A Inhibitors
106 Anti-Retroviral Agents
107 Vitamins
108 Calciferol
109 Hepatitis C Antibodies
110 HIV Antibodies
111 Antiviral Agents

Interventional clinical trials:

(showing 98, show less)
# Name Status NCT ID Phase Drugs
1 Double-blind, Double-dummy, Randomised, Crossover, Multicentre Phase IV Clinical Study Comparing the Effect of Ursofalk 500 mg Tablets od Versus Ursofalk 250 mg Capsules od on Liver Enzymes in the Treatment of Primary Biliary Cirrhosis Completed NCT01510860 Phase 4 UDCA (Ursodeoxycholic acid);UDCA (Ursodeoxycholic acid)
2 Abatacept For The Treatment Of Primary Biliary Cirrhosis With An Incomplete Biochemical Response To Ursodeoxycholic Acid Completed NCT02078882 Phase 4
3 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
4 A Phase 4, Double-Blind, Randomized, Placebo-Controlled Study Evaluating the Pharmacokinetics and Safety of Obeticholic Acid in Patients With Primary Biliary Cholangitis and Moderate to Severe Hepatic Impairment Recruiting NCT03633227 Phase 4 Obeticholic Acid (OCA);Placebo
5 A Phase 4, Double-Blind, Randomized, Placebo-Controlled, Multicenter Study Evaluating the Effect of Obeticholic Acid on Clinical Outcomes in Patients With Primary Biliary Cholangitis Recruiting NCT02308111 Phase 4 Obeticholic Acid (OCA);Placebo
6 Mechanism of Sorafenib Resistance in Patients With Advanced Hepatocellular Carcinoma Recruiting NCT02733809 Phase 4 Sorafenib
7 Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis: a Randomized Control Study Unknown status NCT02823353 Phase 3 Fenofibrate;UDCA
8 Fuzhenghuayu in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02916290 Phase 3 Fuzhenghuayu;UDCA
9 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
10 Efficacy and Security of Bezafibrate in Patients With Primary Biliary Cirrhosis Without Biochemical Response to Ursodeoxycholic Acid: A Randomized, Double-blind, Placebo-controlled Trial Unknown status NCT02937012 Phase 3 Bezafibrate;Ursodeoxycholic Acid;Placebo (for Bezafibrate)
11 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02916641 Phase 3 Fuzhenghuayu;UDCA
12 METformin in Intrahepatic Cholestasis of Pregnancy (METRIC) Study Unknown status NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
13 Effectiveness of Ursodeoxycholic Acid Use in the Prevention of Gallstone Formation After Sleeve Gastrectomy: A Prospective Randomized Multicenter Placebo-Controlled Trial Unknown status NCT02721862 Phase 3 Ursodeoxycholic Acid;Placebo
14 A Phase 3, Double Blind, Placebo Controlled Trial and Long Term Safety Extension of Obeticholic Acid in Patients With Primary Biliary Cirrhosis Completed NCT01473524 Phase 3 Obeticholic Acid (OCA);Placebo
15 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
16 A Randomized, Double-blinded, Double-dummy, Parallel-controlled and Multicenter Clinical Trial to Investigate Safety and Efficacy of Tauroursodeoxycholic Acid Capsules in Treatment of Adult Primary Biliary Cirrhosis Completed NCT01857284 Phase 3 Tauroursodeoxycholic Acid Capsules;Ursodeoxycholic Acid Capsules
17 Randomized Controlled Pilot Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
18 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
19 Phase III Randomized, Double-Blind, Placebo-Controlled Study of Low-Dose Oral Methotrexate Versus Colchicine for Primary Biliary Cirrhosis Completed NCT00004748 Phase 3 colchicine;methotrexate;ursodiol
20 Phase III Trial of Tetrathiomolybdate (TM) in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
21 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
22 Evaluate the Efficacy And Safety Of TUDCA Compare UDCA In The Treatment Of Cholestatic Liver Disease-PBC by A Randomized,Double-Blind,Double Dummy,Parallel-Controlled,Multicenter Trial and The Consecutive Treatment By TUDCA Completed NCT01829698 Phase 3 tauroursodeoxycholic;ursodeoxycholic acid
23 Multicenter, Randomized, Double-blind Placebo Controlled Trial of Bezafibrate for the Treatment of Primary Biliary Cirrhosis in Patients With Incomplete Response to Ursodesoxycholic Acid Therapy. Completed NCT01654731 Phase 3 Bezafibrate;placebo
24 A Phase 3 Study to Evaluate the Place of Budesonide in the Treatment of GVHD Completed NCT00128739 Phase 3 Budesonide
25 Estudio clínico Fase III Para Evaluar la Eficacia terapéutica en Pacientes Mexicanos Con Dislipidemia Mediante el Uso vía Oral de L-Carnitina + Atorvastatina Comparado Con Atorvastatina Active, not recruiting NCT03696940 Phase 3 L-Carnitine 500Mg Oral Tablet + Atorvastatin 10 mg;Atorvastatin 10mg
26 An Open Label Long-Term Study to Evaluate the Safety and Tolerability of Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) Suspended NCT03301506 Phase 2, Phase 3 Seladelpar 2 mg Capsule;Seladelpar 5 mg Capsule;Seladelpar 10 mg Capsule
27 Double-blind, Randomised, Placebo-controlled, Multi-centre Phase III Clinical Study Comparing the Combination of Ursodeoxycholic Acid Capsules Plus Budesonide Capsules to Ursodeoxycholic Acid Capsules Plus Placebo in the Treatment of Primary Biliary Cirrhosis Terminated NCT00746486 Phase 3 budesonide;budesonide placebo
28 A Phase I/II, Open Label, Multicenter, Pilot Dose Escalation Study to Evaluate the Safety, Tolerability and Pharmacodynamics of FFP104 in Subjects Previously Diagnosed With Primary Biliary Cirrhosis (PBC) Unknown status NCT02193360 Phase 1, Phase 2 FFP104
29 Phase 1/2 Study of UC-MSC Treatment for Evaluation the Efficacy and Safety in Patients With Primary Biliary Cirrhosis Unknown status NCT01662973 Phase 1, Phase 2
30 A Randomized Controlled Clinical Trial on the Efficacy and Safety of Fenofibrate Combined With Ursodeoxycholic Acid in PBC Patients With an Incomplete Biochemical Response to UDCA Unknown status NCT02965911 Phase 1, Phase 2 Fenofibrate;UDCA
31 Open-Labelled, Multicenter Phase II Study of Rituximab in Patients With Steroid-Refractory Chronic Graft-Versus-Host Disease Unknown status NCT00472225 Phase 2 Rituximab
32 An 8-week, Dose Ranging, Open Label, Randomized, Phase 2 Study With a 44-week Extension, to Evaluate the Safety and Efficacy of MBX-8025 in Subjects With Primary Biliary Cholangitis (PBC) and an Inadequate Response to or Intolerance to Ursodeoxycholic Acid (UDCA) Unknown status NCT02955602 Phase 2 MBX-8025 2 mg Capsule;MBX-8025 5 mg Capsule;MBX-8025 10 mg Capsule
33 A Phase 2, Randomized, Double Blind, Placebo Controlled, Parallel Group, Multiple Center Study to Evaluate the Safety, Tolerability, and Pharmacodynamic Activity of NGM282 in Combination With Ursodeoxycholic Acid (UDCA) Administered for 28 Days in Patients With Primary Biliary Cirrhosis Completed NCT02026401 Phase 2
34 A Phase 2, Multi-Center Study to Evaluate Three Doses of NGM282 Administered for 24 Weeks in Patients With Primary Biliary Cirrhosis (PBC) Who Have Completed Study 13-0103 Completed NCT02135536 Phase 2
35 A Study of INT 747 (6α-ethyl Chenodeoxycholic Acid (6-ECDCA)) in Combination With Ursodeoxycholic Acid (URSO®, UDCA) in Patients With Primary Biliary Cirrhosis Completed NCT00550862 Phase 2 INT-747;Ursodeoxycholic Acid (URSO);Placebo
36 A Phase 2, Randomized, Double-blind, Placebo-controlled Study to Evaluate LUM001, an Apical Sodium-dependent Bile Acid Transporter Inhibitor (ASBTi) in Combination With Ursodeoxycholic Acid (UDCA) in Patients With Primary Biliary Cirrhosis Completed NCT01904058 Phase 2 LUM001;Placebo;Ursodeoxycholic Acid
37 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
38 Pilot Study of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
39 A Study of INT-747 (6-ECDCA) Monotherapy in Patients With Primary Biliary Cirrhosis Completed NCT00570765 Phase 2 Placebo;INT-747;INT-747
40 A Pilot Study of Pentoxifylline for the Treatment of Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline
41 Open-Label Pilot Investigation of Moexipril for the Treatment of Primary Biliary Cirrhosis (PBC) Completed NCT00588302 Phase 2 Moexipril
42 Effects of Rituximab (Rituxan) on B Cell and AMA Response in Patients With Primary Biliary Cirrhosis Completed NCT00364819 Phase 1, Phase 2 rituximab
43 A Phase 2 Clinical Trial Investigating the Effects of Obeticholic Acid on Lipoprotein Metabolism in Subjects With Primary Biliary Cirrhosis Completed NCT01865812 Phase 2 obeticholic acid (OCA)
44 A Phase 2, Multi-center, Randomized, Double-blind, Placebo-controlled, Parallel-group Study Evaluating the Efficacy and Safety of Ustekinumab in Subjects With Primary Biliary Cirrhosis Who Had an Inadequate Response to Ursodeoxycholic Acid (UDCA) Completed NCT01389973 Phase 2 ustekinumab 90 mg;ustekinumab 45 mg;ustekinumab 180 mg;Placebo
45 A Randomised, Double-blind, Placebo-controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Repeat Doses of GSK2330672 Administration in Patients With Primary Biliary Cirrhosis (PBC) and Symptoms of Pruritus Completed NCT01899703 Phase 2 GSK2330672;Placebo;Ursodeoxycholic acid
46 A Multi-part, Randomized, Double-blind, Placebo-controlled Study to Assess the Safety, Tolerability and Efficacy of Tropifexor (LJN452) in Patients With Primary Biliary Cholangitis Completed NCT02516605 Phase 2 Part 1: LJN452;Part 1: Placebo;Part 2: LJN452 Dose level 1;Part 2: Placebo;Part 2: LJN452 Dose level 2
47 A Double-Blind, Randomized, Placebo-Controlled Clinical Trial to Assess the Efficacy and Safety of Oral GKT137831 in Patients With Primary Biliary Cholangitis Receiving Ursodeoxycholic Acid and With Persistently Elevated Alkaline Phosphatase Completed NCT03226067 Phase 2 GKT137831;Placebo oral capsule
48 B-Cell Depleting Therapy (Rituximab) as a Treatment for Fatigue in Primary Biliary Cirrhosis Completed NCT02376335 Phase 2
49 Obeticholic Acid Treatment in Patients With Bile Acid Diarrhoea: an Open-label, Pilot Study of Mechanisms, Safety and Symptom Response. Completed NCT01585025 Phase 2 Obeticholic acid
50 Safety and Efficacy of Human Unbilical Cord Derived-mesenchymal Stem Cells Treatment for Patients With Decompensated Liver Cirrhosis Recruiting NCT03945487 Phase 2
51 A Phase 2, Prospective, Multicenter, Randomized, Double-blind, Placebo-controlled Study to Evaluate Safety, Tolerability and Efficacy of Saroglitazar Magnesium in Patients With Primary Biliary Cholangitis (EPICS ) Active, not recruiting NCT03112681 Phase 2 Saroglitazar magnesium 2 mg;Saroglitazar magnesium 4 mg;Placebo Oral Tablet
52 A Phase IIa, Randomized, Double-blind, Placebo-controlled Study of TQA3526 in the Treatment of Naive or Previously Treated PBC Patients Not yet recruiting NCT04278820 Phase 2 TQA3526;Placebo to match TQA3526
53 An Open Label Single Arm Study to Investigate the Safety and Efficacy of Multiple Administrations of NI-0801, a Fully Human Anti-CXCL10 Monoclonal Antibody in PBC Patients With an Incomplete Response to Ursodeoxycholic Acid Terminated NCT01430429 Phase 2 NI-0801
54 A 12-week, Double-blind, Randomized, Placebo-controlled, Phase 2 Study, to Evaluate the Effects of Two Doses of MBX-8025 in Subjects With Primary Biliary Cirrhosis (PBC) and an Inadequate Response to Ursodeoxycholic Acid (UDCA) Terminated NCT02609048 Phase 2 Placebo Comparator;Experimental: Seladelpar / MBX-8025 50 mg;Experimental: Seladelpar / MBX-8025 200 mg
55 An Exploratory, Phase IIa Study to Demonstrate the Safety and Efficacy of A4250 in Patients With Primary Biliary Cirrhosis and Cholestatic Pruritus Terminated NCT02360852 Phase 2 A4250
56 S-Adenosyl Methionine for Symptomatic Treatment of Primary Biliary Cirrhosis Terminated NCT00125281 Phase 2 S-adenosyl-methionine (SAMe) capsules
57 Randomized Controlled Study of Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Withdrawn NCT01141296 Phase 2 fenofibrate;placebo
58 An Open-label, Multicenter Extension Study to Evaluate the Long-term Safety and Tolerability of LUM001, an Apical Sodium-dependent Bile Acid Transporter Inhibitor (ASBTi), in Patients With Primary Biliary Cirrhosis Withdrawn NCT02321306 Phase 2 LUM001
59 Phase I Clinical Trial, Randomized, Controlled, to Evaluate the Efficacy and Safety of Therapy With Allogenic Mesenchymal Stem Cells From Bone Marrow for Patients With Refractory Primary Biliary Cirrhosis Unknown status NCT01440309 Phase 1 ursodeoxycholic acid
60 Antibodies Screening in Multiple Sclerosis as a Part of an International Survey in Autoimmune Diseases. Unknown status NCT00230204 Phase 1
61 Phase 1 Study of Intrahepatic Reinfusion of Highly Purified CD133+ Stem Cells in Patients With End-Stage Liver Disease Unknown status NCT01025622 Phase 1
62 Open Label, Single Dose Study Of The Pharmacokinetics Of Tigecycline In Adult Subjects With Primary Biliary Cirrhosis Completed NCT00406237 Phase 1 tigecycline
63 Modafinil in the Treatment of Fatigue in Patients With Primary Biliary Cirrhosis Completed NCT00943176 Phase 1 Modafinil
64 Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis Completed NCT00004842 Phase 1 budesonide
65 A Single-Center, Open-Label, Dose-Escalation Study of Cyclic Adenosine Monophosphate (AMP)-Response Element-binding (CREB)-Binding Protein (CBP) / β-Catenin Inhibitor OP-724 in Patients With Primary Biliary Cholangitis (Phase I) Recruiting NCT04047160 Phase 1 OP-724
66 Safety and Efficiency of Mesenchymal Stem Cell in Treating Pneumonia Patients Infected With COVID-19 Recruiting NCT04252118 Phase 1
67 Examination of the Metabolic Effects of Direct Bile Salt Delivery to the Ileum in Humans Recruiting NCT03201952 Phase 1 Ursodeoxycholic Acid;Placebos
68 Non-myeloablative Allogeneic Hematopoietic Stem Cell Transplantation in Patients With Primary Biliary Cirrhosis: A Phase I Study Withdrawn NCT00393185 Phase 1
69 Measurement of Fibrinogen in Patients With Systemic Inflammatory Response Syndrome, Sepsis, Chronicle Liver Disease or After Lysis on Intensive Care Units Unknown status NCT01169168
70 Identification of the Genetic Variants Responsible for Primary Biliary Cirrhosis (PBC) Unknown status NCT00145964
71 to Investigate the Maternal Serum IL-17 Levels in Pregnant Women With Intrahepatic Cholestasis of Pregnancy Unknown status NCT01898832
72 Family Studies in Primary Biliary Cirrhosis Unknown status NCT00242125
73 A Study Of Metabolic Factors And Efficacy Of Pentoxifylline Versus Pioglitazone In Lean And Obese Nash (Non-Alcoholic Steatohepatitis) Patients. Unknown status NCT00681733 Pioglitazone;Pioglitazone;Pentoxifylline
74 Differential Gene Expression in Liver Tissue and Blood From Individuals With Chronic Viral Hepatitis With or Without a Complicating Hepatoma or Autoimmune Liver Disease Unknown status NCT00160940
75 Impact of a High-protein High-fiber Diet on the Nutritional Status of Patients With Primary Biliary Cirrhosis Completed NCT01603199
76 The Health Burden of Primary Biliary Cirrhosis (PBC) in Switzerland Completed NCT02846896
77 Randomized Controlled Pilot Study of Highly Active Anti-Retroviral Therapy for Patients With Primary Biliary Cirrhosis Completed NCT01614405 Truvada and Kaletra
78 Identification of Inflammatory and Fibrotic Biomarkers in PBC and NAFLD Patients Completed NCT02477462
79 An Observational Study to Evaluate the Efficacy of Nalfurafine Hydrochloride in Patients With Primary Biliary Cholangitis Completed NCT02659696
80 A Clinical Study About Gut Microbes and Metabolic Group in Different PBC Patients for UDCA Response Completed NCT03590886
81 Portal Vein Thrombosis Relevance On Liver Cirrhosis: Italian Venous Thrombotic Events Registry Completed NCT01470547
82 Effect of Obeticholic Acid (INT-747, Intercept) on the Hepatobiliary Transport of Bile Acids in Patients With PBC Examined by 11C-cholyl-sarcosine PET/CT Completed NCT03253276 Early Phase 1 Obeticholic acid;Placebos
83 Mesenchymal Stem Cell Transplantation for Refractory Primary Biliary Cholangitis, a Randomized Double-blind Placebo Controlled Trial Recruiting NCT03668145
84 The Role of the Vitamin D Receptor Gene Polymorphisms in Hepatocarcinogenesis in Cirrhotic Patients Infected With Chronic Hepatitis C Virus Recruiting NCT02461979
85 Evaluation of Patients With Liver Disease Recruiting NCT00001971
86 Patterns and Outcome of Autoimmune Related Chronic Liver Disease : Retrospective Study Recruiting NCT03898414
87 Macrophage Activation Marker sCD163 in PBC Patients - Assessment of Treatment Response to UDCA Recruiting NCT02931513
88 Macrophage Activation Marker sCD163 in PBC Patients - Assessment of Disease Severity and Prognosis Recruiting NCT02924701
89 Sulfation of Bile Acids as a Biomarker for Hepatobiliary Diseases Recruiting NCT01200082
90 EXerCise Intervention in cholesTatic LivEr Disease: The EXCITED Study Recruiting NCT04265235
91 Incidence and Mortality of Hepatocellular Carcinoma in HIV-infected Individuals in an Asian Population Recruiting NCT03702998
92 Role of Obeticholic Acid in the Patients of NAFLD With Raised ALT- A Randomized Clinical Control Trial Study Recruiting NCT03836937 Obeticholic acid
93 Understanding the Genetic Predisposition to the Development of Primary Biliary Cirrhosis (PBC). Active, not recruiting NCT01161953
94 Clinical Trial on the Effect of Sublimated Mare Milk Supplement in Patients With Biliary Cholangitis Active, not recruiting NCT03665519 Ursodeoxycholic Acid
95 A Resource of Blood and Other Biospecimens of Patients With Cholestatic Liver Disease and Unaffected Individuals Enrolling by invitation NCT03445585
96 The Assesment of Matrix Metalloproteinase-1 Genotypes Polymorphism as a Risk Factor for Hepatocellular Carcinoma in Chronic Hepatitis C Patients With Liver Cirrhosis Not yet recruiting NCT03722628
97 Applicability of Liver Transplantation in Alcoholic Hepatitis Suspended NCT01768715
98 Open-Label Pilot Study Evaluating Oral Budesonide in the Treatment of Patients With Primary Biliary Cirrhosis and Overlap Features of Autoimmune Hepatitis. Withdrawn NCT00587119 Budesonide

Search NIH Clinical Center for Biliary Cirrhosis, Primary, 1

Genetic Tests for Biliary Cirrhosis, Primary, 1

Anatomical Context for Biliary Cirrhosis, Primary, 1

MalaCards organs/tissues related to Biliary Cirrhosis, Primary, 1:

40
Liver, B Cells, Bone Marrow, Bone, Breast, Testes

Publications for Biliary Cirrhosis, Primary, 1

Articles related to Biliary Cirrhosis, Primary, 1:

(showing 48, show less)
# Title Authors PMID Year
1
Dense fine-mapping study identifies new susceptibility loci for primary biliary cirrhosis. 56
22961000 2012
2
Variants at IRF5-TNPO3, 17q12-21 and MMEL1 are associated with primary biliary cirrhosis. 56
20639879 2010
3
Genome-wide meta-analyses identify three loci associated with primary biliary cirrhosis. 56
20639880 2010
4
Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. 56
19458352 2009
5
Identification of novel alleles at a polymorphic microsatellite repeat region in the human NRAMP1 gene promoter: analysis of allele frequencies in primary biliary cirrhosis. 56
10712108 2000
6
Primary biliary cirrhosis. 56
8900092 1996
7
Primary structure analysis and lamin B and DNA binding of human LBR, an integral protein of the nuclear envelope inner membrane. 56
8157662 1994
8
Familial primary biliary cirrhosis associated with impaired concanavalin A-induced lymphocyte transformation in relatives. Two family studies. 56
1735358 1992
9
Isolation and characterization of cDNA encoding a human nuclear antigen predominantly recognized by autoantibodies from patients with primary biliary cirrhosis. 56
2258622 1990
10
Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis. 56
2167346 1990
11
Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase. 56
3174635 1988
12
Coexistence of CREST syndrome and primary biliary cirrhosis. Serological studies of two cases. 56
3172118 1988
13
Genetic abnormalities of immunoregulation in primary biliary cirrhosis. 56
6222651 1983
14
Familial occurrence of primary biliary cirrhosis associated with hypergammaglobulinemia in descendants: a family study. 56
6965374 1980
15
Towards a biotechnological platform for the production of human pro-angiogenic growth factors in the green alga Chlamydomonas reinhardtii. 61
31822980 2020
16
Plasma membrane expression of G protein-coupled estrogen receptor (GPER)/G protein-coupled receptor 30 (GPR30) is associated with worse outcome in metachronous contralateral breast cancer. 61
32302351 2020
17
Complete Genome Sequences of Bacillus Bacteriophages Wes44 and Carmen17. 61
30938704 2019
18
Development of a Multiplex PCR Test System for the Determination of a Transgene Based on the pBC1 Plasmid and Its Derivatives for the Expression of Recombinant Proteins in Mus musculus Milk. 61
31201639 2019
19
Odor mitigation and bacterial community dynamics in on-site biocovers at a sanitary landfill in South Korea. 61
29957505 2018
20
An AANAT/ASMT transgenic animal model constructed with CRISPR/Cas9 system serving as the mammary gland bioreactor to produce melatonin-enriched milk in sheep. 61
28273380 2017
21
A novel and highly specific phage endolysin cell wall binding domain for detection of Bacillus cereus. 61
26043681 2015
22
Bacteriophage PBC1 and its endolysin as an antimicrobial agent against Bacillus cereus. 61
25595773 2015
23
Validation of a recombinant human bactericidal/permeability-increasing protein (hBPI) expression vector using murine mammary gland tumor cells and the early development of hBPI transgenic goat embryos. 61
24289868 2013
24
Effective induction of pblac1 laccase by copper ion in Polyporus brumalis ibrc05015. 61
23332833 2013
25
Optimization of The Electroporation Conditions for Transfection of Human Factor IX into The Goat Fetal Fibroblasts. 61
23577306 2013
26
The construction and expression of lysine-rich gene in the mammary gland of transgenic mice. 61
22577831 2012
27
In vitro evaluation of a mammary gland specific expression vector encoding recombinant human lysozyme for development of transgenic dairy goat embryos. 61
22526423 2012
28
Complete genome sequence of Bacillus cereus bacteriophage PBC1. 61
22570248 2012
29
[Construction and identification of mammary expressional vector for cDNA of human lactoferrin]. 61
21650051 2011
30
Transient expression of chicken antimicrobial peptides by mouse mammary carcinoma cells C127. 61
20937033 2010
31
Development of a luciferase-based reporter system to monitor Bifidobacterium breve UCC2003 persistence in mice. 61
18816375 2008
32
Improved cloning vectors for bifidobacteria, based on the Bifidobacterium catenulatum pBC1 replicon. 61
18539807 2008
33
Functional analysis of the pBC1 replicon from Bifidobacterium catenulatum L48. 61
17704917 2007
34
Screening for plasmids among human bifidobacteria species: sequencing and analysis of pBC1 from Bifidobacterium catenulatum L48. 61
16930703 2007
35
Selection of donor nuclei in somatic cell-mediated gene transfer using a co-transfection method. 61
17077582 2007
36
Construct synthetic gene encoding artificial spider dragline silk protein and its expression in milk of transgenic mice. 61
17364439 2007
37
Molecular evidence that the extracellular cutinase Pbc1 is required for pathogenicity of Pyrenopeziza brassicae on oilseed rape. 61
12795380 2003
38
A cryptic plasmid, pAO1, from a compost bacterium, Bacillus sp. 61
11272838 2001
39
Transgenic Japanese lawngrass (Zoysia japonica Steud.) plants regenerated from protoplasts. 61
30736568 1998
40
Study of stability of recombinant plasmids during the continuous culture of Bacillus stearothermophilus NUB3621 in nonselective medium. 61
18634046 1997
41
Characterization, sequence and mode of replication of plasmid pNB2 from the thermophilic bacterium Clostridium thermosaccharolyticum. 61
9003300 1996
42
Updating prognosis in primary biliary cirrhosis using a time-dependent Cox regression model. PBC1 and PBC2 trial groups. 61
8253362 1993
43
Structural organization of a cryptic plasmid, pMA1, from Microcystis aeruginosa f. aeruginosa Kützing. 61
7764220 1993
44
Structural organization of pBC1, a cryptic plasmid from Bacillus coagulans. 61
1729254 1992
45
Stable transformation of sorghum cell cultures after bombardment with DNA-coated microprojectiles. 61
24221592 1991
46
Characterization of gram-positive broad host-range plasmids carrying a thermophilic replicon. 61
1908113 1991
47
Microelectrode assessment of chloride-conductive properties of cortical collecting duct. 61
6465322 1984
48
Plasmid transformation in Bacillus subtilis: effects of insertion of Bacillus subtilis DNA into plasmid pC194. 61
6267418 1981

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